Manifestasi di Bidang Gigi-Mulut dari Kelainan Hematologi Jumat.pptx

Kelainan Hemato Oral mulut

1. Manifestasi di Bidang Gigi- Mulut Kelainan Hematologi

2. normal blood cells

3. HEMATOLOGIC (BLOOD) DISEASES Bleeding Disorders Platelet Function Coagulation Factor Patelet Deficiency = Thrombocytopenia/Thrombocytopathia Red Blood Cell (RBC) Disorders Anemia White Blood Cell (WBC) Disorders Leukopenia Leukemia (Lymphoma)

4. Kelainan RBC Iron-deficiency Anemia Megaloblastic Anemia and Pernicious Anemia Aplastic Anemia Thalassemia Sickle-cell Anemia Polycythemia G-6PD Deficiency

5. Definition Anemia is a decrease in the number of RBCs, hemoglobin (Hb) content, or hematocrit (Hct), or A condition in which the number of red blood cells (and consequently their oxygen - carrying capacity) is insufficient to meet the body’s physiologic need.

6. WHO criteria

7. Mechanisms of anemia

8. Pathophysiology

9. Laboratory Diagnostic strategy of anemia

10. Testing

11. 1. Complete blood count and RBC indices Hemoglobin, RBC count, WBC count, number of platelets, Hematocrit RBC indices: MCV, MCH, MCHC Determine RDW The RBC indices can help indicate the mechanism of anemia and narrow the number of possible causes Microcytic hypochromic Normocytic normochromic Macrocytic

12. RBC WBC PLT

13. 2. Etiology of anemia based on mean corpuscular volume (MCV) of RBC

14. 3. Reticulocyte count percentage of reticulocytes (normal range, 0.5 to 1.5%)

15. 4. Peripheral smear H ighly sensitive , M ore accurate than automated technologies Red cells are examined for (1) size, (2) shape, (3) color, (4) inclusions and (5) other abnormalities

16. 5. Bone marrow aspiration and biopsy direct observation and assessment of RBC precursors abnormal maturation (dyspoiesis) of blood cells and the amount, distribution, and cellular pattern of iron content usually not indicated in the evaluation of anemia and are only done when one of the following conditions is present: Unexplained anemia More than one cell lineage abnormality (ie, concurrent anemia and thrombocytopenia or leukopenia) Suspected primary bone marrow disorder (eg, leukemia, multiple myeloma, aplastic anemia, myelodysplastic syndrome, metastatic carcinoma, myelofibrosis)

17. Anemia Defisiensi Besi

18. Metabolisme besi dan patogenesis defisiensi besi

19. Hitung sel darah lengkap dan morfologi eritrosit www.drsarma.in

20. Manifestasi Klinis Anemia Clinical: Weakness Fatigue Pallor Decreased Oxygen Carrying Capacity of Blood Oral Features: Pallor Bald Tongue  loss of filiform and fungiform papillae on the dorsum of tongue

21. Manifestasi Oral Anemia Defisiensi Besi When severe, angular cheilitis can occur Pallor of oral tissues Erythematous, smooth, painful tongue Filiform and fungiform papillae disappear as with all anemias

22. Megaloblastic Anemia and Pernicious Anemia

23. Pemeriksaan laboratorium anemia megaloblastik

24. Sequence of Development of Megaloblastic Anemias Normal serum B12 levels 160-900 pmol/L Normal serum folat is 7-25 nmol/L

25. Megaloblastic Anemia and Pernicious Anemia

26. Neutrophil Lobe Count Hypersegmented neutrophils is essentially pathognomonic for megalob an and inexpensive The rule of reporting: At least five 5-lobed PMNs per 100 WBC At least one 6-lobed PMNs per 100 WBC Average of Lobe count on 100 neutrophil > 3.4

27. Manifestasi Klinis Anemia defisiensi vit B12 Oral signs are similar to other anemias plus: Oral manifestation possibilities are glossitis (beefy red tongue) or stomatitis (generalized burning or soreness). Red and smooth dorsum of the tongue with areas of ulcerations.

28. Aplastic Anemia

29. How to diagnose Aplasia usually presents with pancytopenia - decreased numbers of erythrocytes, leukocytes, and platelets in the peripheral blood. Aplasia must be confirmed by bone marrow biopsy. Other hematopoietic disorders can present with pancytopenia and must be distinguished from aplastic anemia. These include paroxysmal nocturnal hemoglobinuria, myelodysplasia, and acute leukemia. 33

30. Note that hypersplenism can cause a pancytopenia, but that the marrow would be hyperplastic, rather than hypocellular as in aplasia. The degree of severity is judged by several factors including: -marrow cellularity < 25% -absolute recticulocyte count < 40,000/ m ml -platelets < 20,000/ m ml -granulocyte count < 500/ml (some use < 200/ m ml) 34

31. 35 A. Normal bone marrow tissue B. Hypoplastic bone marrow

32. Manifestasi Klinis Anemia aplastik Infeksi Perdarahan spontan Purpuric spot, petekie

33. Thalassemia

34. Thalassemia ThalassemiaThalassemia (Mediterranean Anemia) A group of inherited disorders of hemoglobin synthesis An autosomal dominant inheritance pattern The heterozygous form may be mildly symptomatic or asymptomatic. The homozygous form is associated with severe hemolytic anemia.

35. Distribution of red blood cell abnormalities worldwide https://en.wikipedia.org/wiki/Hemoglobin_E

36. Clinical Features and Oral Manifestations of Thalassemia Yellow skin & pallor, fever, malaise, and weakness The face includes prominent cheekbones, depression of the bridge of the nose, a prominent maxilla, and protrusion or flaring of maxillary anterior teeth. Radiographs of skull may show marked vertical striation (hair on end appearance) Skull x-ray in a 5-year old boy with thalassemia major Marked vertical striations give the appearance of hair standing on end

37. Dampak talasemia mayor 41

38. Dampak talasemia mayor Appearance of typical thalassemic facies 42 BUTUH TRANSFUSI DARAH SEUMUR HIDUP !!!

39. Pewarisan talasemia Most Likely Case tobe Missed Due to Lack of Screening and Both Parents Asymptomatic Carriers

40. Sickle Cell Anemia

41. Sickle Cell anemia An inherited blood disorder Occurs before age 30 and is more common in women than in men The red blood cells develop a sickle shape when there is decreased oxygen This can be triggered by exercise, exertion, administration of a general anesthetic, pregnancy, or even sleep.

42. Dental Complications of Sickle Cell Disease Dental Caries Dental Erosions Infractions Hypodontia Malocclusions Pulp Necrosis Abnormal Trabecular spacing Infection https://www.omicsonline.org/open-access/dental-complications-of-sickle-cell-disease-jimds-2-152.php?aid=32134

43. Polycythemia

44. Polycythemia Characterized by an increase in the number of circulating red blood cells May be absolute or relative The three forms of polycythemia are Polycythemia Vera Secondary Polycythemia Relative Polycythemia

45. Polycythemia Oral Manifestations of Polycythemia The oral mucosa may appear deep red to purple The gingiva may be edematous and bleed easily Submucosal petechiae, ecchymosis, and hematoma formation may be present.

46. G-6PD Deficiency

47. Glucose-6-phosphate dehydrogenase (G6PD) deficiency uncommon inherited enzyme deficiency characterized by hemolytic anemia caused by the inability of erythrocytes to detoxify oxidizing agents such as drugs, infectious diseases, or fava bean ingestion Avoid oxidant drugs eg asprin, phenacetin, sulphonamides,- 1 chloramphenicol, and vit K

48. Terima kasih